ABSTRACT
BACKGROUND: Pemphigus is a life-threatening autoimmune blistering disease mediated by autoantibodies against adhesion molecule of the skin. Its concurrence with systemic and organ-specific autoimmune disease was described in case reports. OBJECTIVES: To evaluate the presence of a broad spectrum of organ-specific and non-organ-specific autoantibodies other than anti-desmoglein antibodies in pemphigus patients. PATIENTS AND METHODS: Serum samples were obtained from 105 pemphigus foliaceus (PF) patients, 51 pemphigus vulgaris (PV) patients and 50 controls. Both indirect immunofluorescence assay and ELISA were used to assess the presence of autoantibodies related to connective tissue diseases, autoimmune hepatitis, vasculitis, rheumatoid arthritis, coeliac disease, diabetes and thyroiditis. RESULTS: Significant difference was observed between the three groups for anti-thyroglobulin antibodies in the pemphigus foliaceus group (18% vs. 4%, P=0.03). A significantly higher occurrence of IgM anti-cardiolipin (P=0.03), IgG anti-reticulin (P=0.01) and IgG anti-gliadin antibodies (P=0.008) were observed in the PV group. Cases with more than four autoantibodies were frequently positives for both anti-desmoglein 1 and anti-desmoglein 3. CONCLUSION: Autoantibodies other than anti-desmoglein antibodies are not rare in pemphigus patients. Clinical and serological follow-up of pemphigus patients with positive autoantibodies are needed to clarify their impact in disease evolution.
Subject(s)
Autoantibodies/blood , Desmogleins/immunology , Pemphigus/immunology , Adult , Aged , Aged, 80 and over , Case-Control Studies , Enzyme-Linked Immunosorbent Assay , Female , Fluorescent Antibody Technique , Humans , Male , Middle Aged , Pemphigus/blood , Radioimmunoassay , Seroepidemiologic StudiesABSTRACT
AIMS: To study by flow cytometry (FCM) the ploidy and the cellular cycle of nodular hidradenoma (NH) and hidradenocarcinoma (HC) and to assess the prognostic utility of this technique in such tumors. METHODS: We studied retrospectively 2 HC and 11 NH one of which was considered as an atypical NH. Monoparametric study by FCM was realized on paraffin-embedded material. The extracted cells were marked by Propidium's lodure and cellular cycle was analyzed by the software Mod-Fit LT. RESULTS: Our study showed eleven 100% diploid profiles, 10 of which had low S-phase varying between 2 and 12%. All of these 11 tumors were NH. S-phase was high (23.79%) in a single case that corresponded to the atypical NH. Two tumors showed aneuploid profiles; these corresponded to the 2 HC. CONCLUSION: The results of the cytometric study suit perfectly to those of the histopathologic examination. FCM could so help to establish the prognosis of these tumors. But further studies are necessary to determine the value of this technique.
Subject(s)
Adenoma, Sweat Gland/pathology , Cell Cycle , Ploidies , Sweat Gland Neoplasms/pathology , Adenoma, Sweat Gland/genetics , Adolescent , Adult , Aged , Aneuploidy , DNA, Neoplasm/genetics , Diploidy , Female , Flow Cytometry , Follow-Up Studies , Humans , Male , Middle Aged , Neoplasm Metastasis , Neoplasm Recurrence, Local/genetics , Neoplasm Recurrence, Local/pathology , Prognosis , Retrospective Studies , S Phase , Sweat Gland Neoplasms/geneticsABSTRACT
Syringocystadenoma papilliferum (SCAP) is a rare cutaneous adnexal neoplasm with variable clinical appearance and characteristic histology. It arises in about one third of cases within a sebaceous hamartoma (SH) and in this case, multiple other benign adnexal neoplasms may be associated with it. We report the clinical and pathological data of 8 cases of syringocystadenoma papilliferum. The age at the time of the diagnosis varied from 3 to 48 years with an average age of 28 years. Three tumors were localized in the scalp, 2 in the trunk, and 3 others of unusual locations: 1 in the eyelid, 1 in the thigh and 1 in the popliteal fossa. Histopathological examination revealed 3 SCAP occurring within SH. The SCAP observed in the eyelid was associated with an apocrine cystadenoma without features of SH. The four others were not associated with any other lesion.
Subject(s)
Adenoma, Sweat Gland/pathology , Sweat Gland Neoplasms/pathology , Adult , Child, Preschool , Female , Humans , Male , Middle Aged , Retrospective StudiesSubject(s)
Cystadenoma/diagnosis , Knee , Sweat Gland Neoplasms/diagnosis , Syringoma/diagnosis , Adult , Humans , MaleABSTRACT
We describe the histomorphometric changes in the superficial peroneal nerve biopsy from 13 patients with xeroderma pigmentosum (XP). The mean age at time of biopsy was 15 yr (range 2.5-24 yr). The clinical examination was normal in the two youngest patients and showed absence of the deep tendon reflexes, with choreo-athetosis in ten patients. In addition, in the three oldest patients there were cerebellar signs, ataxia and Babinski signs. The nerve biopsy showed an age-dependent decrease of myelinated fibres, which was mild in the youngest and severe in the oldest patients. This was associated with rare acute axonal degeneration, sparse axonal regeneration, rare axonal atrophy and few onion bulb formations. These findings suggest a neuropathic process. This neuronal degeneration seems to be a progressive and stereotyped phenomenon in XP.
